Summary of the pathogenesis of CAPS. Even without the trigger, inflammasome formation process takes place in Cryopyrin mutants (1a and 1b). Procaspase 1 turns into Caspase 1 by the inflammasome action (2). IL-1β is maturated by Caspase 1 (3). IL-1β binds to the receptor (4a). IL-1β signaling cascade followed by inflammation is activated by the binding of IL-1β (4b). Signaling by the Receptor-IL-1β complex leads to further transcription of proIL-1β and thus increase in Caspase 1 independent IL-1β levels (4c). Signaling by the Receptor-IL-1β complex also stimulates the antagonist production (4d). Binding of antagonist to the receptor does not result in active IL-1β signaling (4e).